We have tested, in a prospective randomized, double-blind, placebo-controlled, crossover, 12-week study, the effects of 2 mg efaroxan, a potent alpha-2 antagonist, given three times per day to 14 patients with progressive supranuclear palsy. Efaroxan did not induce any significant change on any motor assessment criteria. The present data do not confirm the assumption that the blockade of alpha-2 receptors might be a useful pharmacologic strategy to improve patients with progressive supranuclear palsy.
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http://dx.doi.org/10.1002/mds.870130411 | DOI Listing |
Gac Med Mex
January 2025
Laboratorio de Reprogramación Celular y Enfermedades Crónico-Degenerativas, Department of Physiology, Universidad Nacional Autónoma de México, Mexico City, Mexico.
Progressive supranuclear palsy (PSP) is a rare, atypical parkinsonism, characterized by the presence of intracerebral tau protein aggregates and determined by a wide spectrum of clinical features. The definitive diagnosis is postmortem and is identified through the presence of neuronal death, gliosis, and aggregates of the tau protein presented in the form of neurofibrillary tangles (MNF) with a globose appearance in regions such as the subthalamic nucleus, the substantia nigra, and the globus pallidus The findings in ancillary imaging studies, as well as fluids biomarkers, are not sufficient to support diagnosis of PSP but are used to rule out similar pathologies because there are still no specific or validated biomarkers for this disease. The current treatment of PSP is focused on reducing symptoms, although emerging therapies seek to counteract its pathophysiological mechanisms.
View Article and Find Full Text PDFBrain Behav
January 2025
Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany.
Background: While automated methods for differential diagnosis of parkinsonian syndromes based on MRI imaging have been introduced, their implementation in clinical practice still underlies considerable challenges.
Objective: To assess whether the performance of classifiers based on imaging derived biomarkers is improved with the addition of basic clinical information and to provide a practical solution to address the insecurity of classification results due to the uncertain clinical diagnosis they are based on.
Methods: Retro- and prospectively collected data from multimodal MRI and standardized clinical datasets of 229 patients with PD (n = 167), PSP (n = 44), or MSA (n = 18) underwent multinomial classification in a benchmark study comparing the performance of nine machine learning methods.
Parkinsonism Relat Disord
January 2025
Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan. Electronic address:
Introduction: Recent studies by us and others have unveiled a frequent coexistence of idiopathic normal pressure hydrocephalus (iNPH) with neurodegenerative movement disorders, including progressive supranuclear palsy (PSP). This study aims to explore the clinical and radiological characteristics of patients with iNPH who also had comorbid with PSP, referred to as iNPHc + PSP.
Methods: We retrospectively analyzed patients with iNPH admitted to our department between 2009 and 2024.
Parkinsonism Relat Disord
January 2025
Department of Radiology, Mayo Clinic, Rochester, MN, USA. Electronic address:
Introduction: White matter (WM) tract degeneration is a characteristic feature of progressive supranuclear palsy (PSP), with longitudinal changes observed in PSP-Richardson's syndrome (PSP-RS). Little, however, is known about the other PSP variants. We assessed cross-sectional and longitudinal WM degeneration across PSP variants using diffusion tractography.
View Article and Find Full Text PDFJ Neurol
January 2025
Centre de Génétique Humaine, Centre Hospitalier Universitaire de Besançon, Besançon, France.
Introduction: The MAPT gene encodes Tau, a protein mainly expressed by neurons. Tau protein plays an important role in cerebral microtubule polymerization and stabilization, in axonal transport and synaptic plasticity. Heterozygous pathogenic variation in MAPT are involved in a spectrum of autosomal dominant neurodegenerative diseases known as taupathies, including Alzheimer's disease, Pick's disease, fronto-temporal dementia, cortico-basal degeneration and progressive supranuclear palsy.
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